[The effect of collagen-normalizing therapy on the incidence and severity of anesthetic and postoperative complications in children with connective tissue syndromes].

A new method of collagen-normalizing therapy has been worked out to prevent specific anesthesia-induced, operative and postoperative complications and to treat some symptoms in children with Ehlers-Danlos and Marfan's syndromes and with non-classified Marfan-like malformations. The technique involves a combined use of beta-adrenoblocker in the age-matched doses and vitamins C/0.03 g/(kg.day)/, B2/0.0004 g/(kg.day)/and B6/0.002 g/(kg.day)/ for 2.5 months before surgery and during the first 2 weeks of the postoperative period. The above therapy reduces the incidence of delayed recovery of the muscular tone and adequate respiration, spontaneous and recurrent pneumothorax, hemorrhagic and gastroenterologic complications, as well as the incidence and severity of intestinal paresis. The indexes of effective collagen-normalizing therapy are as follows: body weight increase, echocardiographic pattern of reduced diameter of the aorta and mitral valve prolapse, normalization of the urinary excretion of total and polypeptide-bound oxyproline. The efficacy of therapy depends on the baseline level of oxyproline excretion.